I fa te hian an thinlunga thisen kalna kawng bulpui (main blood vessel) a tikhawlo em? Interrupted Aortic Arch (IAA) chungchang hi a awlsam zawngin i zir dawn nia.

I naute piang thar i en hian i hlimna chu sawifiah theih loh a ni lawm ni? Chutih rual chuan, chu nun tlemte atan chuan i thinlungah mawhphurhna lian tak a awm a ni. A châng chuan naupang tê tête natna ṭhenkhat i hriat hian hlauhna leh lungkhamna nasa tak i neih hi thil pangngai a ni. Vawiin hian thinlung natna khirh tak, nausen piang thara lo awm thei, mahse a tlem hle a, damdawi lam pan vat ngai a ni tih kan sawi dawn a ni. Hetiang dinhmun hi Interrupted Aortic Arch, a tawi zawngin (IAA) an ti a.

A awlsam zawngin sawi ila, Interrupted Aortic Arch (IAA) hi eng nge ni?

Hei hi hrethiam tur chuan kan thinlung hnathawh dan hi han thlir hmasa ila. Kan thinlung hi tui pump ang maiin han ngaihtuah teh. He pump hian main chamber pali a nei a. A chung lamah pahnih leh a hnuai lamah pahnih. Taksain a hman tawh leh oxygen level hniam tak, a sen deuh thin thisen hi upper right chamber-ah a lut a. Chuta tang chuan lower chamber-ah a kal a, lungah a pump lut a ni. Lung-ah chuan he thisen hian oxygen a nei tha hle a, rawng sen mawi tak a nei thin.

Tunah chuan he oxygenated red blood hi thinlung veilam chamber chunglam ah a lut ta a ni. Chuta tang chuan veilam hnuai lam chamber-ah a kal a, kan taksaa thisen kalna kawng lian ber leh pawimawh ber aorta hmangin taksa pum puiah a pump chhuak a ni. He aorta hi kan tuikhuah atanga in chhung zawnga tui phurtu main pipe system ang a ni. Candy cane ang maia siam a ni.

Chuvangin, naute Interrupted Aortic Arch (IAA) tih natna neia thil thleng chu thisen kalna bulpui, aorta chu a kim lo tihna a ni. Khawi emaw laiah chuan daltu a awm a, chu chu gap chhe tawh ang mai a ni. A awlsam zawngin, tui pipe lian ber chu a laiah a chhe vek a ni. Chumi avang chuan thinlung hian engtiang pawhin nasa takin pump mah se, oxygen tamna thisen tha tak chu taksa hnuai lam ah a kal lo.

Engtin nge i naute hian he natna hi a nei tih i hriat theih ang? Eng nge a lan chhuah dan?

IAA vei naute hian an pian hnu darkar engemaw zat emaw, ni engemaw zat emaw chhungin natna lanchhuahna an nei tan tlangpui. Nu leh pa kan nih angin hei hi in hriat chian a pawimawh hle.

I naute zingah heng chhinchhiahna pakhat emaw a aia tam emaw i hmuh chuan doctor pan nghal tur a ni a, emaw, i naute chu damdawi ina Emergency Treatment Unit (ETU)-ah hruai nghal tur a ni

Engvangin nge naute chungah hetiang hi a thlen?

Hetiang thil i hriat hian nu leh pa i nih angin i rilruah zawhna lo lang hmasa ber chu, "Engvangin nge kan naute chungah hetiang thil hi a lo thlen? Thil dik lo kan ti em?"

A awlsam zawngin sawi ila, he dinhmun hi naute chu nausen pum chhunga a lo thanglian lai a ni a, chu chu chromosome hmun tlemte tak tak hloh vang a ni a, chu chuan thinlung leh thisen kalna kawng lian ber berte siamna a nghawng a ni.

IAA vei naute hian DiGeorge syndrome an nei fo thin.Genetic condition dang DiGeorge syndrome tih nen a awm thei bawk. Hei hi a chunga kan sawi tawh ang khan chromosome khata a then hloh vang a ni. DiGeorge syndrome vei naupangte hian natna an vei fo thei a, hmaiah pawh danglamna engemaw zat ( hmui hrual ang chi) an nei thei bawk.

IAA nena inzawm thinlung chhiatna dangte

IAA vei naute hian thinlung lama harsatna dang pakhat emaw a aia tam emaw an nei fo thin. A tam ber pakhat chu Ventricular Septal Defect (VSD) a ni.

• Ventricular Septal Defect (VSD): A awlsam zawngin sawi ila, hei hi thinlung pumping chamber lian pahnih (ventricles) inkar wall (septum)-a khur a ni. Hei hi a tlangpuiin chhiatna ni mahse, VSD hi IAA vei naute tan chuan hun eng emaw chen atan nunna chhanhimtu a ni thei. Ngaihtuah teh, aorta atanga thisen a luang chhuak thei lo lai hian aorta veilam a oxygenated thisen thenkhat chu he khur atang hian dinglam lamah a luang lut thei a, chuta tang chuan lung lam panna thisen kalna kawng kaltlangin taksa hnuai lam chuan thisen supply engemaw zat an hmu thei a ni. Kawngpui lian a inkhar lai hian a lehlam panna tur bypass te tak te a awm ang mai a ni. Mahse hei hi hun rei lote chhunga chinfelna chauh a ni.

Diagnosis leh enkawl dante chu engte nge ni?

He natna hi a rang thei ang bera hriat leh enkawl a pawimawh.

Diagnosis tih a ni

Hetiang dinhmun hi Echocardiogram hmanga hmuhchhuah a ni tlangpui . ‘Echo test’ kan ti mai mai a ni. Hei hi thinlung scan ang mai a ni. He natna nei lo test hian thinlung pindan, valve leh thisen kalna kawngte pianzia leh hnathawh dan chiang takin a hmu thei a ni. A châng chuan, naute chu naute pum chhunga a la awm laiin scan tih hmangin pawh a hmaa hriat theih a ni.

Tihdamna

IAA enkawlna chu operation a ni . Amaherawhchu, naute chu zai tura a inpeih hma chuan doctor ten naute nunna chhanhim nan thil dang an ti hmasa ang.

1. Step khatna - Temporary Duct hawn reng:

Nausen piang thar apiang hian thinlung, aorta leh lung lama thisen phurtu tube , Ductus Arteriosus tia koh, inzawmna chu hun eng emaw chen atan an nei vek a ni. Hei hi naute pum chhunga a awm lai hian a pawimawh hle. He tube hi a pian atanga darkar tlemte chhungin a mah chauhin a khar thin.

Mahse IAA vei nauteah chuan aorta a chhe tawh avangin taksa chhunga oxygenated thisen luang chhuak tur kawng dang awmchhun chu Ductus Arteriosus kaltlangin a ni. Chuvangin doctor-te tih hmasak ber chu naute chu Prostaglandin E1 tih damdawi an pe a, chu chuan chu temporary tube chu khar lovin a hawng reng a ni. Hei hian naute taksaa thisen supply chu operation hma loh chuan a vawng reng thei a ni.

2. Step hnihna - Surgery: 1.1.

Naute dinhmun a nghet tawh chuan specialist surgeon-te chuan surgery an nei thin. Hei hian a huam a ni:

• Aorta tawp chhe tawh pahnih chu la a, inzawm khawm a, thisen kalna kawng tluantling, tihbuai loh siam.
• Prostaglandin pek hmanga hawn tawh Ductus Arteriosus hun eng emaw chen kha khar.
• VSD (heart wall-a khur) a awm chuan he surgery neih chhung hian khar a ni bawk ang.

Naute chu operation hnuah engtin nge a awm ang?

Nu leh pate tan chuan an nau piang thar hian nunna atana hlauhawm natna a nei tih hriat ai chuan tuar harsa zawk a awm lo. Mahse kan sawi tawh atang hian he natna hi damdawi lam pan nghal vat chuan hlawhtling taka enkawl theih a ni tih i hmu thei.

IAA operation hnua naupang hriselna hi a tha thei hle . Statistics atanga a lan dan chuan zai a nih atanga kum 10 hnuah pawh naupang 81% chu an la dam a ni. Chumi awmzia chu hlawhtlinna rate a sang hle tihna a ni.

Mahse hei hi emergency a ni. A natna lan chhuah dan a hriat veleh naute chu damdawi in intensive care unit (ICU) -ah dah luh nghal tur a ni a, enkawl tan nghal tur a ni. Surgery hnuah pawh naute chu clinic-ah hruai chhunzawm zel a, a tul anga test neih tir zel a pawimawh hle a, doctor-te thurawn angin.